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Localized scleroderma and regional inflammatory myopathy.

Saša A Zivković1, William Freiberg2, David Lacomis3

  • 1Neurology Service, Department of Veterans Affairs, Pittsburgh, PA 15240, USA; Department of Neurology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, USA.

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Localized scleroderma rarely involves inflammatory myopathy. This study highlights regional inflammatory myopathy in localized scleroderma, emphasizing the need for prompt diagnosis and treatment of myositis.

Keywords:
Inflammatory myopathyLinear sclerodermaLocalized sclerodermaMorpheaPerimyositis

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Area of Science:

  • Rheumatology
  • Neurology
  • Dermatology

Background:

  • Localized scleroderma is a rare connective tissue disease.
  • Inflammatory myopathy is an uncommon complication of localized scleroderma.

Observation:

  • Two new cases of regional inflammatory myopathy associated with localized scleroderma were identified.
  • A review of 10 previously reported cases revealed a predilection for upper extremity involvement.
  • Patients presented with regional muscle weakness, pain, or atrophy.

Findings:

  • Serum creatine kinase levels were typically normal or mildly elevated.
  • Histopathology frequently showed perimysial inflammation and plasma cell infiltration.
  • Muscle biopsy confirmed inflammatory myositis.

Implications:

  • Inflammatory myopathy should be considered in localized scleroderma patients with regional muscle symptoms.
  • Early diagnosis via muscle biopsy is crucial.
  • Treatment with anti-inflammatory and/or immunosuppressive therapies is indicated for confirmed myositis.